Home About Shands For Healthcare Professionals Newsroom ShandsConnect Index
Search:


Skin layers
Skin layers


Ectodermal dysplasia

Definition:

Ectodermal dysplasia is a hereditary condition characterized by abnormal development of the skin, hair, nails, teeth, and sweat glands.



Alternative Names:
Anhidrotic ectodermal dysplasia

Causes, incidence, and risk factors:

There are many different types of ectodermal dysplasia. Multiple genes have been discovered that cause ectodermal dysplasias. The most common form of ectodermal dysplasia is linked to the X chromosome and usually affects men. Another form of the disease affects men and women equally.

Patients with ectodermal dysplasia may have absent or decreased sweating because of a lack of  sweat glands. Children with the disease may have difficulty controlling fevers. Mild illness may produce extremely high fevers, because their skin can't sweat and control temperature properly.

Affected adults are unable to tolerate a warm environment and require special measures to maintain a normal body temperature.

Patients may have chronic nasal infections with foul-smelling discharge and increased lung infections.

The skin is thin with light coloring. Hair may be absent or very thin. Teeth develop abnormally, and many teeth are missing.




Review Date: 11/21/2005
Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2004 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Illustrated Health Encyclopedia

En Español

Pregnancy Health Center

Shands Careguides

Surgeries and Procedures

Shands HealthCast